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Spinal neurofibromas are the most prevalent group of spinal tumors. They occur sporadically
or in association with Neurofibromatosis type-1 (NF1, von Recklinghausen disease). A
neurofibromas developing a dumbbell tumor is a situation which is quite often seen. Surgical
intervention is indicated when myelopathy and motor deficits develop in the case of paraspinal
neurofibromas. The goal of surgery is total removal of the tumor. However, in selected cases partial removal of the tumor with adequate spinal cord decompression can be preferred to prevent
severe complications such as vertebral artery injury. We present a case of neurofibroma with neck
and shoulder pain and dumbbell tumor formation at the level of C1 that was in close relation with
the vertebral artery. Possible surgical interventions are discussed.
Key words: Dumbbell formation, spinal neurofibroma, surgical treatment, upper servical
area
J Nervous Sys Surgery 2008; 1(3):190-194
st Servikal Omurilik Blgesinde Gzlenen Kum Saati eklinde Nrofibroma: Olgu Sunumu
Spinal nrofibromlar spinal tmrlerin nemli bir ksmn oluturur. Sporadik yada
Nrofibromatozis tip-1 (NF1, von Recklinghausen hastal) ile birlikte grlr. Nrofibromann
Dummbell tmr eklinde geliim gstermesi olduka sk grlen bir durumdur. Paraspinal
nrofibroma olgularnda myelopati veya motor defisit gelitii durumlarda cerrahi endikasyon
doar. Cerrahide ama tmrn total karmdr. Ancak seilmi vakalarda, vertabral arter yaralanmas gibi ar komplikasyonlardan kanmak amacyla, yeterli spinal kord dekompresyonu
salayacak ekilde parsiyel karm seilebilir. Bu makalede, C1 dzeyinde vertabral arter ile
yakn ilikide olan, boyun ve omuz ars ile prezente olan dummbbell nrofibroma olgusu sunulmutur. Secilebilecek cerrahi giriim ekilleri tartlmtr.
Anahtar kelimeler: Cerrahi tedavi, kum saati formasyonu, spinal nrofibroma, st servikal
blge
J Nervous Sys Surgery 2008; 1(3):190-194
191
DISCUSSION
Of the paraspinal neurofibromas; 72 % were
with intradural extramedullar localization, where
as 14 % were with extradural, 13 % were with
dumbbell formation and 1 % was with intramedullary localizations (5,7,16). A neurofibroma in the
spinal canal, invading the peripheral segment of
the nerve by extending out of the intervertebral
foramen and presenting itself with a dumbbell
tumor is quite common. In the series of cases
presented by Seppala et al., 21 (66 %) of 32
neurofibromas demonstrated both intradural and
extradural tumor components, and 17 tumors
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resection should be preferred in dumbbell neurofibroma cases that cause compression of the
spinal cord. As the aim of partial resection is to
resolve the symptoms, the extent of surgical
treatment is shaped according to the clinical
picture of the patient. Our patient presented with
myelopathic findings and, therefore, decompressive excision of the tumor was planned. In
decompression surgery, intradural component of
the tumor that compressed the spinal cord was
excised but foraminal and extradural components were left. Decompressive surgery is a
partial resection that carries with itself the risk
of recurrence and surgery may be needed
(5,8,9,10,11)
.
Dumbbell tumors with significant dissemination
into the paraspinal region may require complex
spinal exposure. Although two-stage operations
may be performed to manage the intraspinal and
paraspinal components separately, a single-stage
procedure is preferable. For cervical tumors, the
VA is another issue to be considered. In most
instances, meningiomas and nerve sheath tumors
receive little blood from the spinal cord and are
attached by few adhesions to the spinal cord.
Most cervical dumbbell tumors can be adequately accessed through a standard laminectomy
and complete unilateral facetectomy. As in our
case, this allows paraspinal access up to 3 cm
from the lateral dural margin. A second-stage
anterior procedure may be required if further
tumor extension is present (2,4,10,12).
The VA is consistently displaced anteromedially
by dumbbell neurofibromas of the cervical
spine. The artery is neither encased nor invaded
by these tumors but is separated from the tumor
capsule by a thin layer of periosteum and perivertebral veins. These tissues serve as an effective and easily developed plane of dissection
that is rarely associated with VA injury. Thus,
because of the low risk of either VA injury or its
potential ischemic consequences, preoperative
angiography and/or test occlusion or early intra-
7.
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